Alexander E L, Moyer C, Travlos G S, Roths J B, Murphy E D
Arthritis Rheum. 1985 Oct;28(10):1146-55. doi: 10.1002/art.1780281011.
We have recently described 2 histopathologic types of inflammatory vascular disease (IVD) in patients with Sjögren's syndrome (SS): neutrophilic IVD (NIVD) and mononuclear IVD (MIVD). Autoimmune MRL/Mp mice, which have many features of SS, spontaneously develop IVD which is histopathologically indistinguishable from that observed in human SS patients. Both MRL/Mp-+/+ and MRL/Mp-lpr/lpr mice develop MIVD which evolves into NIVD and results in decreased survival; the transition to NIVD is accelerated by the lpr gene. The presence of the lpr gene on other genetic backgrounds does not result in a similar acceleration of IVD and associated decreased survival. Thus, the spontaneous autosomal recessive mutation lpr appears to modulate the development of IVD in a strain of mice with an underlying propensity for vasculitis. Based on our observations on IVD in SS patients and MRL/Mp mice, we propose a new model which may enhance our understanding of the immunopathogenesis of IVD in connective tissue disease.
我们最近描述了干燥综合征（SS）患者炎症性血管疾病（IVD）的两种组织病理学类型：嗜中性粒细胞性IVD（NIVD）和单核细胞性IVD（MIVD）。具有SS许多特征的自身免疫性MRL/Mp小鼠会自发发生IVD，其组织病理学与人类SS患者中观察到的无法区分。MRL/Mp-+/+和MRL/Mp-lpr/lpr小鼠都会发生MIVD，MIVD会演变为NIVD并导致存活率降低；lpr基因会加速向NIVD的转变。在其他遗传背景上存在lpr基因不会导致IVD出现类似的加速以及相关的存活率降低。因此，自发的常染色体隐性突变lpr似乎在具有潜在血管炎倾向的小鼠品系中调节IVD的发展。基于我们对SS患者和MRL/Mp小鼠中IVD的观察，我们提出了一个新模型，该模型可能会增进我们对结缔组织病中IVD免疫发病机制的理解。
